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Increased risk of tumor in DM1 is not related to exposure to common lifestyle risk factors

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An Erratum to this article was published on 25 February 2016

Abstract

Recent studies documented an increased risk of neoplasm in patients with myotonic dystrophies (DM). Yet, none of these studies evaluated the contribution of common cancer risk factors in such observation. In this study, we included a cohort of patients (n = 255) with an established molecular diagnosis of DM type 1 (DM1), and who receives their treatment in one of the four centers with recognized expertise in neuromuscular disorders in Rome. We estimated the prevalence of benign and malignant tumors, and assessed if lifestyle factors and/or specific disease features would be associated to their occurrence. Overall, 59 benign tumors in 54 patients and 19 malignant tumors in 17 patients were diagnosed. The most common malignant neoplasms were cancers of the skin (31.6 %), thyroid (21.0 %), ovary (10.5 %), and breast (10.5 %). Uterine fibroid was the most common benign tumor (37.6 %) in women, while pilomatricoma was the most common in men (28.6 %). Age at enrollment (OR = 1.02, 95 % CI 1.00–1.05), and female gender (OR = 5.71, 95 % CI 2.90–11.22) were associated with tumor development in DM1 patients, while thyroid disorders was associated with malignant tumors only in women (OR = 5.12, 95 % CI 1.35–19.37). There was no association between tumor development and evaluated lifestyle factors. In conclusion, the lack of association between common cancer risk factors and tumor development in DM1 support a pathogenic link between tumors and DM1 itself, emphasizing the need for a systematic surveillance. Our observation of an association between thyroid diseases in women and cancer development needs confirmation.

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Acknowledgments

The work of Dr. Emanuele Leoncini was supported by Fondazione Veronesi. Dr. Shahinaz Gadalla is supported by the intramural program of the National Cancer Institute, Bethesda, MA.

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Authors and Affiliations

  1. Department of Geriatrics, Neuroscience and Orthopedics, Università Cattolica del Sacro Cuore, Largo F. Vito 1, 00168, Rome, Italy

    Maria Laura Ester Bianchi, Anna Modoni & Gabriella Silvestri

  2. Institute of Public Health, Section of Hygiene, UCSC, Rome, Italy

    Emanuele Leoncini & Stefania Boccia

  3. IRCSS Fondazione Santa Lucia, Rome, Italy

    Marcella Masciullo

  4. Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD, USA

    Shahinaz M. Gadalla

  5. Department of Systems Medicine (Neurology), University of Rome Tor Vergata, Rome, Italy

    Roberto Massa, Emanuele Rastelli & Chiara Terracciano

  6. Department of Neurosciences, Mental Health and Sensory Organs (NESMOS), Sapienza University of Rome, Rome, Italy

    Giovanni Antonini & Elisabetta Bucci

  7. Unità Operativa Complessa Neurologia e Neurofisiopatologia, Azienda Ospedaliera San Camillo Forlanini, Rome, Italy

    Antonio Petrucci

  8. Unit of Medical Genetics, Center for Neuromuscular and Neurological Rare Diseases, S. Camillo Forlanini Hospital, Rome, Italy

    Sandro Costanzi

  9. Fondazione Don Carlo Gnocchi Onlus, Milan, Italy

    Massimo Santoro

Authors
  1. Maria Laura Ester Bianchi
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  2. Emanuele Leoncini
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  3. Marcella Masciullo
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  4. Anna Modoni
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  5. Shahinaz M. Gadalla
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  6. Roberto Massa
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  7. Emanuele Rastelli
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  8. Chiara Terracciano
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  9. Giovanni Antonini
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  10. Elisabetta Bucci
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  11. Antonio Petrucci
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  12. Sandro Costanzi
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  13. Massimo Santoro
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  14. Stefania Boccia
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  15. Gabriella Silvestri
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Corresponding author

Correspondence to Gabriella Silvestri.

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The authors declare no conflict of interest.

Ethical standards

The study was designed in accordance with the guidelines of the Ethical Committees of the Università Cattolica del Sacro Cuore, University of Tor Vergata, University La Sapienza and Azienda Ospedaliera S.Camillo Forlanini, Rome Italy, and in agreement with the Declaration of Helsinki.

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Bianchi, M.L.E., Leoncini, E., Masciullo, M. et al. Increased risk of tumor in DM1 is not related to exposure to common lifestyle risk factors. J Neurol 263, 492–498 (2016). https://doi.org/10.1007/s00415-015-8006-y

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  • DOI: https://doi.org/10.1007/s00415-015-8006-y

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